Cardiac fibroma

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A ~~cardiac~~Cardiac fibroma, ~~(also~~also known as cardiac fibromatosis, are benign congenital cardiac ~~fibromatosis, fibrous hamartoma, or fibroelastic hamartoma) represents a collection of fibroblasts interspersed among large amounts of collagen (some authors believe~~tumours that ~~this tumour is a hamartoma rather than a neoplasm)~~usually manifest in children.

Epidemiology

Cardiac fibroma is a tumour that primarily affects children (most cases is detected in infants or in utero). They are the second most common benign primary cardiac tumour in children after cardiac rhabdomyoma and second commonest fetal cardiac tumour ².

Clinical presentation

Cardiac fibromas are often associated with arrhythmias which can be a presenting feature.

Pathology

Microscopic appearance

Cardiac fibromas ~~almost always arise in the ventricles, most commonly in the ventricular septum and left ventricular free wall~~represents a collection of fibroblasts interspersed among large amounts of collagen (some authors believe that this tumour is a hamartoma rather than a neoplasm).

Macroscopic appearance

They are well-defined, solitary, intramyocardial lesions with smooth margins and usually large, with a mean diameter of ~5 cm and may obliterate the ventricular cavity. They almost always arise in the ventricles, most commonly in the ventricular septum and left ventricular free wall. Unlike many primary cardiac tumours, fibromas usually have no foci of cystic change, haemorrhage, or necrosis. Calcification is a common findings.

Associations

There is an increased prevalence of cardiac fibromas in Gorlin syndrome.

Radiographic features

CT

On CT, cardiac fibromas are homogeneous soft-tissue masses that are usually sharply marginated, but also can be infiltrative. Dystrophic calcification is common.

MRI

Due to their dense, fibrous nature, the tumours are usually homogeneous and hypointense on T2-weighted MR images and isointense relative to muscle on T1-weighted images.

~~Cardiac fibromas~~ They often demonstrate little or no contrast material enhancement.

Differential diagnosis

rhabdomyoma
- also seen in young patients
rhabdomyosarcoma
- large and heterogeneous mass
- invasive
myxoma
- intracavitary rather than intramural
metastases
- known primary neoplasm
- less likely in young patients
- multifocal masses

-<p>A<strong> cardiac fibroma</strong> (also known as cardiac fibromatosis, fibrous hamartoma, or fibroelastic hamartoma) represents a collection of fibroblasts interspersed among large amounts of collagen (some authors believe that this tumour is a hamartoma rather than a neoplasm).</p><h4>Epidemiology</h4><p>Cardiac fibroma is a tumour that primarily affects children (most cases is detected in infants or in utero). They are the second most common benign <a href="/articles/primary-cardiac-tumours">primary cardiac tumour</a> in children after cardiac rhabdomyoma and second commonest <a href="/articles/fetal-cardiac-tumours">fetal cardiac tumour</a> <sup>2</sup>.</p><h4>Clinical presentation</h4><p>Cardiac fibromas are often associated with arrhythmias which can be a presenting feature.</p><h4>Pathology</h4><p>Cardiac fibromas almost always arise in the ventricles, most commonly in the ventricular septum and left ventricular free wall.</p><h5>Macroscopic appearance</h5><p>They are well-defined, solitary, intramyocardial lesions with smooth margins and usually large, with a mean diameter of ~5 cm and may obliterate the ventricular cavity. Unlike many primary cardiac tumours, fibromas usually have no foci of cystic change, haemorrhage, or necrosis. Calcification is a common findings.</p><h5>Associations</h5><p>There is an increased prevalence of cardiac fibromas in <a href="/articles/gorlin-goltz-syndrome-1">Gorlin syndrome</a>.</p><h4>Radiographic features</h4><h5>CT</h5><p>On CT, cardiac fibromas are homogeneous soft-tissue masses that are usually sharply marginated, but also can be infiltrative. Dystrophic calcification is common.</p><h5>MRI</h5><p>Due to their dense, fibrous nature, the tumours are usually homogeneous and hypointense on T2-weighted MR images and isointense relative to muscle on T1-weighted images.</p><p>Cardiac fibromas often demonstrate little or no contrast material enhancement.</p>
+<p><strong>Cardiac fibroma, </strong>also known as c<strong>ardiac fibromatosis</strong>, are benign congenital cardiac tumours that usually manifest in children. </p><h4>Epidemiology</h4><p>Cardiac fibroma is a tumour that primarily affects children (most cases is detected in infants or in utero). They are the second most common benign <a href="/articles/primary-cardiac-tumours">primary cardiac tumour</a> in children after cardiac rhabdomyoma and second commonest <a href="/articles/fetal-cardiac-tumours">fetal cardiac tumour</a> <sup>2</sup>.</p><h4>Clinical presentation</h4><p>Cardiac fibromas are often associated with arrhythmias which can be a presenting feature.</p><h4>Pathology</h4><h5>Microscopic appearance</h5><p>Cardiac fibromas represents a collection of fibroblasts interspersed among large amounts of collagen (some authors believe that this tumour is a hamartoma rather than a neoplasm).</p><h5>Macroscopic appearance</h5><p>They are well-defined, solitary, intramyocardial lesions with smooth margins and usually large, with a mean diameter of ~5 cm and may obliterate the ventricular cavity. They almost always arise in the ventricles, most commonly in the ventricular septum and left ventricular free wall. Unlike many primary cardiac tumours, fibromas usually have no foci of cystic change, haemorrhage, or necrosis. Calcification is a common findings.</p><h5>Associations</h5><p>There is an increased prevalence of cardiac fibromas in <a href="/articles/gorlin-goltz-syndrome-1">Gorlin syndrome</a>.</p><h4>Radiographic features</h4><h5>CT</h5><p>On CT, cardiac fibromas are homogeneous soft-tissue masses that are usually sharply marginated, but also can be infiltrative. Dystrophic calcification is common.</p><h5>MRI</h5><p>Due to their dense, fibrous nature, the tumours are usually homogeneous and hypointense on T2-weighted MR images and isointense relative to muscle on T1-weighted images. They often demonstrate little or no contrast material enhancement.</p><h4>Differential diagnosis</h4><ul>
+<li>
+<a title="Cardiac rhabdomyoma" href="/articles/cardiac-rhabdomyoma">rhabdomyoma</a><ul><li>also seen in young patients</li></ul>
+</li>
+<li>
+<a title="Cardiac rhabdomyosarcoma" href="/articles/cardiac-rhabdomyosarcoma">rhabdomyosarcoma</a><ul>
+<li>large and heterogeneous mass</li>
+<li>invasive</li>
+</ul>
+</li>
+<li>
+<a title="Cardiac myxoma" href="/articles/cardiac-myxoma">myxoma</a><ul><li>intracavitary rather than intramural</li></ul>
+</li>
+<li>
+<a title="Cardiac metastases" href="/articles/secondary-cardiac-neoplasms">metastases</a> <ul>
+<li>known primary neoplasm</li>
+<li>less likely in young patients</li>
+<li>multifocal masses </li>
+</ul>
+</li>
+</ul>